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Key pointsLoss of the cytoskeletal protein dystrophin leads to muscle degeneration in Duchenne muscular dystrophy. The mdx mouse lacks dystrophin but muscle disease is mild, in part because upregulation of utrophin compensates for dystrophin. Evidence suggests that Ca2+ entry through mechanosensitive (MS) ion channels contributes to disease pathogenesis, but the role of utrophin in regulating Ca2+...
• Mechanosensitive (MS) channels in skeletal muscle are heteromeric proteins composed of TRPV4 and other subunits. Experiments investigated subconductance fluctuations associated with gating of independent subunits.
• MS channels open by passing through subconductance states and fluctuate between at least four subconductance levels.
• MS channels in mdx muscle spend ∼60% of the time at smaller subconductance...